Benign childhood focal epilepsies *yRenzo Guerrini and *Simona Pellacani *Pediatric Neurology Unit and Laboratories, Children’s Hospital A. Meyer-University of Florence, Florence, Italy; and yIRCCS Fondazione Stella Maris, Pisa, Italy SUMMARY The idiopathic focal epilepsies comprise a group of syndromes characterized by focal-onset seizures for which there is no detectable structural brain abnormality and for which there is a proposed functional mechanism for the epilepsy and electro- encephalography (EEG) abnormalities. This group includes benign rolandic epilepsy (BRE), benign epilepsy with occipital paroxysms (both early onset and late-onset types), idiopathic photosensi- tive occipital lobe epilepsy, and some less well- defined syndromes. The limits of the early onset idiopathic occipital epilepsy syndrome are not clear, and perhaps this entity represents part of a larger syndrome group of ‘‘autonomic’’ age- related epilepsies. The term ‘‘idiopathic’’ implies absence of a structural brain lesion and a genetic propensity to seizures. The term ‘‘benign’’ implies that the epileptic seizures are easily treated or require no treatment, show remission with- out sequelae with ultimate and definitive remis- sion before adulthood, do not have severe or exceedingly disturbing seizures, and have no asso- ciated serious intellectual or behavioral distur- bances. It may be that a syndrome is benign only when it can be recognized early with reasonable certainty, thereby avoiding unnecessary investiga- tions, overtreatment, and lifestyle restrictions. Although BRE has such characteristic clinical and EEG features to make early recognition possible, this is less constantly so in the other focal idio- pathic epilepsy syndromes, where the term ‘‘benign’’ may be inappropriate. Mild and selective neuropsychological impairment may occur even in those with typical syndromes but it is unclear whether such selective deficits outlast the active phase of epilepsy. Sometimes the clinical course may be complicated by obvious cognitive and lan- guage impairments. In such cases, the term benign is obviously inappropriate, even when seizures are rare. In most patients with the typical focal idiopathic epilepsy syndromes, medication is not necessary. KEY WORDS: Benign rolandic epilepsy, Idio- pathic, Occipital epilepsy, Focal epilepsy, Children. Idiopathic focal epilepsies comprise a group of syn- dromes characterized by focal-onset seizures where there is no structural brain abnormality and for which there is likely to be a functional mechanism for the epilepsy and electroencephalography (EEG) abnormalities (Dalla Bernardina et al., 1992; Loiseau et al., 1992). This group includes several syndromes: benign rolandic epilepsy (BRE), benign epilepsy with occipital paroxysms (both early onset and late onset types), idiopathic photosensitive occipital lobe epilepsy, and some less well-defined syn- dromes. The term ‘‘idiopathic’’ implies absence of a brain lesion and a genetic propensity to seizures (Commission on ILAE, 1989). The term ‘‘benign’’ implies that the epi- leptic seizures are easily treated or require no treatment, show remission without sequelae with ultimate and defini- tive remission before adulthood, do not have severe or exceedingly disturbing seizures, and have no associated serious intellectual or behavioral disturbances. It also implies that early recognition is possible with reasonable certainty (Engel, 2001). Although BRE has such charac- teristic clinical and EEG features to make early recogni- tion possible, this is less constantly so in the other focal idiopathic epilepsy syndromes, where the term ‘‘benign’’ may be inappropriate. Seizures are usually, but not always, closely related to sleep. They remit spontaneously after a few years and there are no associated neurologic signs or cognitive impairment. Some children may have mild and selective neuropsychological impairment but this does not usually outlast the period of active seizures. Certain seizure types Address correspondence to Renzo Guerrini, Neuroscience Depart- ment, Children's Hospital A. Meyer, Viale Pieraccini 24, 50139 Florence, Italy. E-mail: r.guerrini@meyer.it Wiley Periodicals, Inc. ª 2012 International League Against Epilepsy Epilepsia, 53(Suppl. 4):9–18, 2012 doi: 10.1111/j.1528-1167.2012.03609.x COMMON CHILDHOOD EPILEPSIES 9